GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans.
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ABSTRACT:
SUBMITTER: Yao Y
PROVIDER: S-EPMC9111091 | biostudies-literature | 2022 Jun
REPOSITORIES: biostudies-literature
Publications
GIMAP6 regulates autophagy, immune competence, and inflammation in mice and humans.
Yao Yikun Y Du Jiang Ping P Chao Brittany N BN Cagdas Deniz D Kubo Satoshi S Balasubramaniyam Arasu A Zhang Yu Y Shadur Bella B NaserEddin Adeeb A Folio Les R LR Schwarz Benjamin B Bohrnsen Eric E Zheng Lixin L Lynberg Matthew M Gottlieb Simone S Leney-Greene Michael A MA Park Ann Y AY Tezcan Ilhan I Akdogan Ali A Gocmen Rahsan R Onder Sevgen S Rosenberg Avi A Soilleux Elizabeth J EJ Johnson Errin E Jackson Peter K PK Demeter Janos J Chauvin Samuel D SD Paul Florian F Selbach Matthias M Bulut Haydar H Clatworthy Menna R MR Tuong Zewen K ZK Zhang Hanlin H Stewart Benjamin J BJ Bosio Catharine M CM Stepensky Polina P Clare Simon S Ganesan Sundar S Pascall John C JC Daumke Oliver O Butcher Geoffrey W GW McMichael Andrew J AJ Simon Anna Katharina AK Lenardo Michael J MJ
The Journal of experimental medicine 20220513 6
Inborn errors of immunity (IEIs) unveil regulatory pathways of human immunity. We describe a new IEI caused by mutations in the GTPase of the immune-associated protein 6 (GIMAP6) gene in patients with infections, lymphoproliferation, autoimmunity, and multiorgan vasculitis. Patients and Gimap6-/- mice show defects in autophagy, redox regulation, and polyunsaturated fatty acid (PUFA)-containing lipids. We find that GIMAP6 complexes with GABARAPL2 and GIMAP7 to regulate GTPase activity. Also, GIMA ...[more]
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